Congenital Cystic Dilatation of the Common Bile Duct as a Cause

of Primary Bile Duct Stone

Yoshiro Matsumoto, MD, Kyoto, Japan

Kotaro Uchida, MD, Kyoto, Japan

Akira Nakase, MD, Kyoto, Japan

lchio Honjo, MD, Kyoto, Japan

Congenital cystic dilatation of the common bile duct is usually considered to be large cystic dilatation that extends from the wall of the common bile duct to fill the right upper quadrant. It is most commonly found in patients in the pediatric age group. However, pa- tients with dilatations of both the whole extrahepatic bile duct and intrahepatic bile ducts or with a dif- fusely fusiform dilatation of the common bile duct have also been reported [l-2].

It has been indicated previously that the typical characteristics of this anomaly on the cholangiogram are dilatations of the common bile duct with nar- rowing of the distal common bile duct below the en- largement [3-51. We [I] divided patients with con- genital cystic dilatation of the common bile duct into two groups based on clinical and anatomic features: those with infant-type cysts and those with adult- type cysts. The infant-type cyst was the typically large cyst and the adult-type cyst was the fusiform or cylindrical cyst which appeared to be’closely re- lated to the formation of primary bile duct stones and the cause of relapsing acute pancreatitis.

The present report presents the anatomic char- acteristics of the biliary passages and the clinical features of patients with the adult-type cyst, and shows the relationship between this variety of cyst and the formation of primary bile duct stones.

Material and Methods

Diagnosis and Case Materials

Between 1970 and 1975,333 patients with benign biliary disease were treated at the First Department of Surgery of Kyoto University Medical Center. Of the 333 patients, 103 had congenital choledochal cysts [I]: 101 patients had Alonso-Lej type I cysts (congenital cystic dilatation of the common bile duct), 1 had type II cyst (congenital diver- ticulum), and 1 had type III cyst (congenital choledocho-

From the First Department of Surgery, Kyoto University Medical School. Sakyo-ku. Kyoto. Japan.

Reprint requests should be addressed to Yoshiro Matsumoto. MD, First Department of Surgery, Kyoto University Medical School, Sakyo-ku, Kyoto, Japan.

346

Figure 1. T-tube choiangiogram of a fifty-two year old woman with an adult-type cyst, demonstrating the f&form dilatation of the entire extrahepatic bile duct with a long common channel. Floating stones are present in the en- iargement. Note that the junction of the cystic duct with the common bile duct is also involved in the enlargement.

The American Journal of Surgery

Primary Bile Duct Stone

Figure 2. T-tube cholangi~ram ot a thirty year old woman with an adult- type cyst and right upper quadrant pain and jaundice but no biliary stone. R shows the anomalous ar- rangement of the pancreaticobillary ductal system.

cele). The 101 Alonso-Lej type I cysts consisted of two subtypes, which we named “infant-type” and “adult-type”

based on the anatomic location on the cholangiogram [I]. The infant-type cyst involved the choledochus and/or the

common hepatic duct and showed saccular dilatation, with a long narrow segment of the bile duct below the enlarge- ment. It was most common in infants or children. The adult-type cyst was fusiform or cylindrical and included the entire extrahepatic bile duct and/or the main intra- hepatic bile ducts. It was most frequent in adults. Eighty-eight patients were classified as having “adult- type” cysts, eighty-five of whom were of adult age. Material for the present report was collected from the records of these eighty-eight patients.

Cholangiographic Findings of the Adult-Type Cysts

The enlargements of the biliary passages in this variety of cyst showed fusiform or cylindrical dilatations. (Figure 1.) A part of the junction of the cystic duct with the cho- ledochus was also involved, but the gallbladder was gen- erally normal. The narrow segment below the enlargement, ranging in length from 1 to 5 cm, was not as long as that in the infant-type cyst, and it was recognized on the cholan-

Figure 3. Endoscopic retrograde cholangiopancreatogram of a fifty-one year okt man with an ad&tvpe cyst, who had suffered from jaundice, right hypochondralgia, and back pain but had no biliary stone. It reveals the long common channel of the pancreaticobiliary ductal system and the secondary radicles of the pancreatic duct branching from the long common channel.

giograms in thirty-one of the eighty-eight patients. The

anatomic arrangement of the pancreaticobiliary system was anomalous and characteristic as well as that in infant type. The distal common bile duct was inserted into the pancreatic duct (Wirsung) at the usually long distance from

the ampulla of Vater. (Figures 2 and 3.) Accordingly, we believe that the narrow segment in this disease should not be regarded as the terminal bile duct but as the pancreatic duct. Dilatation of the pancreatic duct caudal to the com- munication was demonstrated in many cases. (Figure 4.) However, two of the eighty-eight patients had separate orifices in the papilla and did not show the communication of the terminal bile duct with the pancreatic duct. (Figure

5.)

Clinical Features

The chief complaints of the eighty-eight patients were abdominal pain, jaundice, and fever. Abdominal pain- that is, epigastric, right hypochondric, or back pain-was the most common symptom and occurred in seventy-nine patients (90 per cent). Jaundice was present in seventy patients (80 per cent) and was accompanied with pain in sixty-five (74 per cent). Fever was rare.

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Matsumoto et al

Figure 4. T-tube cholangiogram of a sixty-one year old woman wtth an ad&-type cyst and repeated bouts of acute pancreatttis and chotangttis, demonstrating the anomalous arrangement of pancreaticobiliary ductal system and di- latation of the distal pancreatic duct. She has no stone in the enlargement.

Of the eighty-eight patients, seventy had gallstones, of whom sixty-seven (96 per cent) had pain, fifty-five (79 per cent) jaundice, twenty-eight (40 per cent) fever and twenty-five (36 per cent) all three symptoms at the same time. Of the eighteen patients without calculi, twelve (67 per cent) had pain, fourteen (83 per cent) jaundice, and one (6 percent) fever. The differentiation of symptoms in pa- tients with and without calculi was not clear; accordingly, the symptoms might be caused by bile stasis and bouts of cholangitis.

Laboratoryfindings in most of the eighty-eight patients showed elevations of serum and urine amylase levels, serum alkaline phosphatase levels, and total bilirubin levels at the time of attacks. During remission, the levels stayed within normal ranges despite the existence of the common bile duct stones. In one sixty-four year old woman with cancer of the body of the pancreas associated with this anomaly, endoscopic retrograde cholangiopancreatography revealed many ovoid stones in the dilated common bile duct and gallbladder, but she did not have a history of attacks of bile

Figure 5. Barium study of a seventy-one year old man with an adult-type cyst and separate orifice of the papilla, who had been asymptomatic from his birth. It shows that duo- denal contents easily passed into the biliary tract.

duct stone or bouts of cholangitis, and the serum alkaline phosphatase levels were always within normal limits. (Figure 6.)

Location, Incidence, and Age Distribution of Biliary Calculi

Locations of the stones in the seventy patients with adult-type cysts are as follows. In forty-eight of the seventy patients, no stones were present in the gallbladder. Four patients had stones only in the gallbladder (3 were cho- lesterin stones); sixteen in the gallbladder and common bile duct (3 were cholesterin stones); two in the gallbladder, common bile duct, and intrahepatic duct; five in the com- mon bile duct and intrahepatic duct; thirty-nine in the common bile duct (2 were cholesterin stones); and four in the intrahepatic duct.

Of the 333 patients with benign biliary disease, 104 had bile duct stones, ninety of which were primary stones.

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Primary Bile Duct Stone

Figure 6. Endoscopic retrograde chotangiopancreato- gram of a sixty-four year old woman with an adutt-type cyst, showing multiple bile duct stones in the common bile duct and interruption of the pancreatic duct due to cancer of the body of the pancreas. She was asymptomatic from birth.

Sixty-six of the ninety patients with primary bile duct stones had adult-type cysts and of the sixty-six, eleven had intrahepatic stones. Six patients with intrahepatic stones showed impaction in the major intrahepatic bile duct,

mainly in the left branch. The stones were the “earthy or stasis stones” of Aschoff [6], except for two which were pure cholesterin stones. Figure 7 shows the relationship between age distribution and the incidence of calculi in the eighty-eight patients. Note that the older the patient, the higher the incidence of this disease, and of those aged thirty years and older, 90 per cent had gallstones.

Surgical Treatment and Results

Details of the treatment for the eighty-eight pa- tients are shown in Table I. Cholecystectomy was carried out in all patients at primary exploration. Of the seventy patients with stones, fifty-two underwent cholecystectomy, stone removal, and T-tube can- nulation as the primary procedure. Twenty-six of the fifty-two underwent reoperation, and five of the re-

Figure 7. Relationship between age distribution of eighty- eight patients with adult-type cysts of congenital cystic dilatation of the common bite duct and the incidence of calculi.

maining twenty-six now have abdominal pain, jaundice, and fever. Similarly, of the eighteen pa- tients who underwent removal of stones and T-tube cannulation as a secondary procedure, nine had ter- tiary explorations, and three of the remaining nine now have abdominal pain, jaundice, and fever. Fur- thermore, of the three patients in whom exploration of the common duct and T-tube cannulation were performed three times, two had tetrexplorations of the biliary passages in which a bypass procedure was carried out.

As a primary procedure, sphincteroplasty was performed in five patients, but two of them were explored two more times. One of these two.was a twenty-five year old woman who had had intrahe- patic stones for ten years and repeated bouts of cholangitis and died of biliary cirrhosis.

None of the thirteen patients who underwent a bypass procedure, choledochoduodenostomy, or choledochojejunostomy primarily required reoper- ation and none had complaints after surgery.

Of the eighteen patients without calculi, eleven underwent choledochojejunostomy with Roux-en-Y anastomosis as a primary procedure and all had satisfactory results. The remaining seven patients underwent T-tube drainage; but after removal of the T tube all complained of abdominal pain or jaundice. Of these seven patients, one underwent reoperation and one reexploration and reoperation. One twenty-nine year old woman with three explorations of the common bile duct had repeated attacks of cholangitis for three years and died of biliary cir- rhosis.

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TABLE I Surgical Treatment for 88 Patients with Adult-Type Cysts -_

First Operation Second Operation Third Operation Fourth Operation

70 Patients with Gallstones Removal of stone Removal of stone

and T tube 52 (5)--c and T tube

Papilloplasty

Papilloplasty Choledocho-

duodenostomy Choledocho-

jejunostomy Removal of stone

and T tube Choledocho-

jejunostomy

/

Removal of stone 18 (3)- and T tube

Choledocho- duodenostomy

Choledocho- jejunostomy

1 (1)

6

Choledocho- l- duodenostomy

t

Choledocho- duodenostomy 1

3- Choledocho-

jejunostomy 1

1

5

2 (1) Choledocho-

duodenostomy 5 Choledocho-

jejunostomy 8

18 Patients without Gallstones

T-tube drainage 6 (4) \

i

Choledocho- Hepatidocho- duodenostomy l- jejunostomy 1 (1)

Choledocho- jejunostomy 1 (1)

Choledocho- jejunostomy 12

Note: Numbers in parentheses indicate the number of patients with complaints after surgery.

Comments

Stones lodged in the bile duct are classified as primary or secondary. Secondary stones are formed in the gallbladder and subsequently pass into the common bile duct. The primary duct stone was named “stasis or earthy stone” and is usually soft and ovoid or conforms to the contour of the common bile duct [6]. The primary duct stones, which are formed in the bile duct, are reported to be more common than the secondary bile duct stones. Madden, Van- derheyden, and Kandalaff [6] clinically analyzed sixty patients with primary bile duct stones and found that 20 per cent did not have stones in the gallbladder and 25 per cent had painless jaundice. In addition, the patients with primary duct stones were subjected to a higher incidence of reoperation than those with secondary bile duct stones. Primary or stasis stones formed in ducts that were widely dilated and were associated with stenosis of the papilla of Vater, but the mechanism for this association was not known. Many authors believe that the primary or

stasis stone forms within the duct, the coexistence of cholestasis and bile infection being the causative agents of bilirubinate stones [6-71. Papillitis is con- sidered the most important cause of bile stasis [8], but it is not considered important in the formation of primary bile duct stones because we have not had a case with continuous bile stasis due to chronic pa- pillitis and even the causal mechanism of chronic papillitis is not clear.

Longmire and Mandiola [9] suggested that in ad- dition to the typically large cystic dilatations of the common bile duct, there might be other varieties of congenital choledochal cyst that could also become symptomatic. They regarded this anomaly as one of the conditions of the common bile duct that was re- sponsible for chronic duct dilatations, sluggish bile flow, stone formation secondary to cholestasis, and repeated bouts of obstruction and cholestasis.

In our series ninety patients had primary duct stones, sixty-six of whom had the adult-type cysts of congenital cystic dilatation of the common bile duct. Most of the others were due to bile duct injury.

The American Journal ot Surgery

The typical morphologic characteristics of this anomaly are recognized as dilatation of the common bile duct with narrowing distal to the enlargement. Both the congenital dilatation of the bile duct and the congenital narrowing of the terminal bile duct are considered to cause bile stasis. Furthermore, this distal narrow segment in patients with stones was shorter than in those without stones, because in the former bile infection may be easily caused. The en- largements in adult-type cysts are fusiform or cy- lindrical and at times are difficult to differentiate from the acquired changes. The discriminating finding between the congenital dilatations and the secondary ones is that a segmental dilatation of the cystic duct is always seen at its junction with the common bile duct in the adult-type cyst.

Longmire and Range1 [IO] considered four causes of hepatic calculi: (1) parasitic infestation of the liver; (2) congenital anomalies of the ductal system; (3) carcinoma of the bile duct; and (4) common duct stasis and obstruction. Glenn and Moody [II] re- ported that intrahepatic calculi encountered in the United States, where parasitic infections are rare, often result from anomalous anatomic variation and distortion of the intrahepatic ductal system.

Except for patients with hepatic calculi due to bile duct injury, all of our patients with intrahepatic bile duct stones had the adult-type cysts with intrahe- patic bile duct dilatation. We believe that the nar- rowing of the intrahepatic duct just proximal to the bifurcation is the cause of stone formation in the in- trahepatic bile duct, and in these cases, the intrahe- patic bile duct stone becomes impacted.

Of the eighteen patients without stones, four were asymptomatic and the stones were found on routine examinations. The remaining fourteen patients had pain and jaundice frequently and fever infrequently. At the time of attack serum amylase level was ele- vated, and at surgery induration was palpated in the head of the pancreas. These findings were thought to be due to bouts of acute pancreatitis secondary to malarrangement of the panceaticobiliary ductal system. This malarrangement is likely due to em- bryonic failure in the development of the pancreatic duct and the common bile duct. By ordinary chol- angiography, the long common channel had been interpreted as the narrowing of the terminal bile duct below the enlargement. However, by endoscopic retrograde cholangiopancreatography, this anatomic anomaly was revealed more finely. We and subse- quently Babbitt, Starshak, and Clemett [12] have indicated that the distal narrow segment below the enlargement was not the common bile duct but the pancreatic duct itself.

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With this type of cyst, intraductal pressure is within normal range in the asymptomatic period

Primary Bile Duct Stone

regardless of the presence of the narrow segment. The edematous swelling of the wall of the narrow distal segment secondary to acute gastroenteritis or pan- creatitis may cause a disturbance of bile flow and an increase in intraductal pressure with pain and jaun- dice. At the same time, this inflammatory stricture may also cause bile reflux into the caudal pancreatic duct or stagnation of pancreatic juice with secondary, relapsing acute pancreatitis as a consequence. Even when the anomalous arrangement of the pancrea- ticobiliary ductal passages is not found on the chol- angiogram, it may be assumed that the patient has the malarrangement.

The purpose of surgical treatment for this type of cyst is to remove the bile stasis in the enlargements; however, mere removal of the stones is inevitably followed by a recurrence. The surgical significance of this malady involves not only the narrowing of the terminal bile duct but also the cystic dilatation above the narrowed segment. Accordingly, since release of the narrow segment only is followed by a recurrence of symptoms, bypass procedures to prevent choles- tasis should also be employed in the operation. Either choledochoduodenostomy or choledochojejunostomy using Roux-en-Y jejunal limb anastomosis has given satisfactory results. The diameter of the stoma must be as large as or larger than that of the dilated com- mon bile duct, and an ovoid portion of the wall must be excised in making the stoma. (Figure 8.)

Figure 8. Photograph of the stoma for choledochojejunos- tomy, demonstrating that the diameter is as large as that of the dilated common bile duct.

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Matsumoto et al

Summary report of three cases: A new aetiological theory based on supposed unequal epithelial proliferation at the stags of the

Of the 101 patients with congenital cystic dilata- tion of the common bile duct, eighty-eight with adult-type cysts are reported on. The association of primary-bile duct stone and adult-type cyst of this anomaly is discussed and found to be higher than previously recognized. In the cases without calculi, abnormal arrangement of the pancreaticobiliary ductal system is indicated to play an important role in the manifestation of the symptom. The anatomic and clinical features and the results of treatment for this type of cyst are reviewed.

physiological epithelial occlusion of the primitive choledo- thus. Gann 30: 601, 1936.

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6. Madden JL, Vanderheyden L, Kandalaff S: The nature and sur- gical significance of common duct stones. Surg Gyneco/ Obstet 126: 3, 1968.

7. Maki T: Pathogenesis of calcium bilirubinate stones; role of E coli, j%glucuronidase and coagulation by inorganic ions, polyelectrolytes and agitation. Ann Surg 164: 90, 1966.

8. Jones SA, Steedman RA, Keller TB, Smith LL: Transduodenal sphincteroplasty (not sphincterotomy) for biliary and pan- creatic disease. Am J Surg 118: 292, 1969.

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References

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2. Tsuchida Y, lshida M: Dilatation of the intrahepatic bile ducts in congenital cystic dilatation of the common bile duct. Surgery 69: 776, 1971.

3. Yotsuyanagi S: Contributions to the aetiology and pathogeny of idiopathic cystic dilatation of the common bile duct with

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352 Tha American Journal of Surgery